Placental site trophoblastic tumor (PSTT) is a rare form of the gestational trophoblastic disease (GTD). Seventy four cases have been reported in the English literature up to now. Based on all this data, the death rate is 20% in PSTT. There are many unknown aspect of PSTT on the principles of diagnosis and on the management as well. In this paper, all of the literature related to PSTT has been reviewed and according to these data, diagnosis, clinical behavior and therapy of PSTT have been discussed.